Rokitansky sequence
Rokitansky Sequence, also known as Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, is a rare disorder that affects the reproductive system of females. It is characterized by the underdevelopment or absence of the vagina and uterus. Women with this condition usually have normal external genitalia and normal ovarian function, which means they can produce eggs and have normal hormone levels, leading to typical development of secondary sexual characteristics such as breast development.
Etiology[edit | edit source]
The exact cause of Rokitansky Sequence is not fully understood, but it is believed to involve genetic and environmental factors. It is considered a sporadic condition, meaning it occurs by chance and is not typically inherited. However, there have been cases with familial clustering suggesting a possible genetic component.
Diagnosis[edit | edit source]
Diagnosis of Rokitansky Sequence is often made during adolescence when a girl fails to menstruate by the age of 16, a condition known as primary amenorrhea. Further medical evaluation, including imaging tests such as MRI (Magnetic Resonance Imaging) or ultrasound, can confirm the absence or underdevelopment of the uterus and vagina.
Treatment[edit | edit source]
Treatment for Rokitansky Sequence focuses on the psychological and physical aspects. Psychological counseling is recommended to help individuals cope with the diagnosis and its implications. Surgical and non-surgical options exist to create a functional vagina. The most common surgical procedure is the creation of a neovagina, while non-surgical methods involve the use of dilators to stretch existing tissue. Fertility options are limited, but with advancements in reproductive technologies, options like surrogacy and adoption are considered.
Epidemiology[edit | edit source]
Rokitansky Sequence affects approximately 1 in 4,500 female births. It is one of the most common causes of primary amenorrhea in females.
Impact[edit | edit source]
The impact of Rokitansky Sequence on an individual's life can be significant. It affects physical health, emotional well-being, and fertility. Early diagnosis and support are crucial in managing the condition effectively.
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Contributors: Prab R. Tumpati, MD
